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ORIGINAL ARTICLE
Korean J Pediatr 2010 November;53(11) :965-970.
doi:https://doi.org/10.3345/kjp.2010.53.11.965
Survival analysis of spinal muscular atrophy type I
Hyun Bin Park (Park HB)1, Soon Min Lee (Lee SM)1, Jin Sung Lee (Lee JS)2, Min Soo Park (Park MS)1, Kook In Park (Park KI)1, Ran Namgung (Namgung R)1, Chul Lee (Lee C)1
1Department of Pediatrics, Yonsei University College of Medicine, Seoul,Korea
2Department of Clinical Genetics, Yonsei University College of Medicine, Seoul, Korea
Copyright © 2010 by The Korean Pediatric Society
ABSTRACT
Purpose : The life expectancy of patients with spinal muscular atrophy (SMA) type I is generally considered to be less than 2 years. Recently, with the introduction of proactive treatments, a longer survival and an improved survival rate have been reported. In this study, we analyzed the natural courses and survival statistics of SMA type I patients and compared the clinical characteristics of the patients based on their survival periods. Methods : We reviewed the medical records of 14 pediatric patients diagnosed with SMA type I during a 9-year period. We examined the demographic and clinical characteristics of these patients, calculated their survival probabilities, and plotted survival curves as on the censoring date, January 1, 2010. We also compared the characteristics of the patients who died before the age of 24 months (early-death, ED group) and those who survived for 24 months or longer (long-survival, LS group). Results : The mean survival time was 22.82.0 months. The survival probabilities at 6 months, 12 months, 18 months, 24 months, and 30 months were 92.9%, 92.9%, 76.0%, 76.0%, and 65.1%, respectively. Birth weight was the only factor that showed a statistically significant difference between the ED and LS groups (P=0.048). Conclusion : In this study, the survival probabilities at 2 years were far greater than expected. Because of the limited number of patients and information in this study, the contribution of improved supportive care on longer survival could not be clarified; this may be elucidated in larger cohort studies.
Keywords: Spinal muscular atrophy | Survival | Survival analysis
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